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成功完成一例患有严重成骨不全症患者的口腔修复治疗,该患者经过广泛的预处理、植骨、牙种植体和固定桥:多学科病例报告。

Successful complete oral rehabilitation of a patient with osteopetrosis with extensive pre-treatments, bone grafts, dental implants and fixed bridges: a multidisciplinary case report.

机构信息

Department of Integrative Medical Biology, Umeå University, Umeå, Sweden.

Department of Pediatrics, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

出版信息

BMC Oral Health. 2023 Nov 28;23(1):940. doi: 10.1186/s12903-023-03707-3.

Abstract

BACKGROUND

Osteopetrosis comprises a group of inherited disorders that are rare and result in abnormal bone structure. Bone remodeling is extremely inhibited because osteoclasts are nonfunctional or lacking. This condition causes overgrowth of bone with disappearance of the bone marrow, leading to aplastic anemia; obstruction of nerve passages in the skull leads to blindness and often hearing impairment. In most cases, osteopetrosis results in oral complications such as tooth deformation, hypomineralization, and delayed or absent tooth eruption. The only curative treatment is hematopoietic stem cell transplantation (HSCT). The main treatment of the oral complications during childhood and adolescence consists in protecting the erupted teeth against caries disease through prophylactic treatment aimed at optimal oral hygiene through frequent regular dental visits throughout life. Many patients with osteopetrosis require major oral rehabilitation to treat complications of the disease. Improved results of HSCT increase the likelihood that dental professionals will encounter patients with osteopetrosis.

CASE PRESENTATION

In this case report, we show that individuals with osteopetrosis who have severe oral complications can be treated successfully if they are treated for osteopetrosis at an early age. The boy had his dental care in pedodontics, and regular multidisciplinary meetings were held for future treatment planning. At the age of 15, he was then referred for rehabilitation. The initial evaluations revealed no further growth in the alveolar bone. The rehabilitation was done stepwise, with extraction of malformed and malpositioned teeth. Initially, the patient received a removable partial denture followed by reconstruction of the width of the alveolar process, titanium implants, temporary fixed bridges, and finally screw-retained titanium-ceramic bridges with titanium frames for the upper and lower jaws.

CONCLUSIONS

The three-year follow-up after loading indicated a stable marginal bone level and optimal oral hygiene as a result of frequent professional oral hygiene care. The patient showed no signs of symptoms from the temporomandibular joint and has adapted to the new jaw relation without any functional or phonetical issues.

摘要

背景

成骨不全症是一组罕见的遗传性疾病,导致骨骼结构异常。破骨细胞功能异常或缺失,导致骨重塑受到极大抑制。这种情况会导致骨过度生长,骨髓消失,导致再生障碍性贫血;颅骨神经通道受阻会导致失明,经常导致听力受损。在大多数情况下,成骨不全症会导致口腔并发症,如牙齿变形、矿化不良以及牙齿延迟或缺失萌出。唯一的治愈方法是造血干细胞移植(HSCT)。儿童和青少年期口腔并发症的主要治疗方法是通过预防性治疗保护已萌出的牙齿免受龋齿,这一治疗旨在通过终身定期口腔检查实现最佳口腔卫生。许多成骨不全症患者需要进行主要的口腔康复治疗来处理疾病的并发症。HSCT 效果的改善增加了牙科专业人员遇到成骨不全症患者的可能性。

病例介绍

在本病例报告中,我们展示了如果在幼年时治疗成骨不全症,患有严重口腔并发症的成骨不全症患者可以成功治疗。该男孩在儿童牙科接受口腔护理,定期举行多学科会议以制定未来的治疗计划。15 岁时,他被转诊进行康复治疗。初始评估显示牙槽骨没有进一步生长。康复治疗分阶段进行,包括拔除畸形和错位的牙齿。最初,患者佩戴了可摘局部义齿,随后重建了牙槽嵴宽度,植入钛种植体,临时固定桥,最后为上下颌安装带钛框架的螺丝固位钛瓷桥。

结论

加载后三年的随访表明,由于频繁的专业口腔护理,边缘骨水平稳定,口腔卫生状况良好。患者没有出现颞下颌关节症状,并且适应了新的颌关系,没有任何功能或语音问题。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/520d/10683162/45b4a8e9cc50/12903_2023_3707_Fig1_HTML.jpg

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